Hyperferritinaemia and macrophage activation in a patient with ...

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Feb 23, 2012 - bility of collagen vascular diseases, especially SS. ... Rheumatology key message ... gressive interstitial lung disease (RP-ILD), especially in.
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metastases, pneumatoceles and Pneumocystis carinii pneumoni [7]. Physicians should also consider the possibility of collagen vascular diseases, especially SS. Additionally, the differential diagnosis of pulmonary involvement in SS includes lymphoma and amyloidosis, and thoracoscopic lung biopsy can be required to obtain a definitive diagnosis. In conclusion, this is the first case of pneumothorax as a presenting manifestation of SS. This case emphasizes the importance of a careful clinical history and examination in the evaluation of pneumothorax. Although rare, SS should be considered in the patient presenting with pneumothorax and multiple lung cysts. Rheumatology key message .

SS should be considered as a cause of spontaneous pneumothorax.

Acknowledgements We would like to thank Dr Yoshinori Kawabata, Department of Pathology, Saitama Cardiovascular and Respiratory Center, Japan, for the valuable comments on lung biopsy findings. Disclosure statement: The authors have declared no conflicts of interest.

Satoshi Watanabe1, Yuichi Tambo1, Yuko Waseda2, Osamu Nishimura3, Shinya Murakami3, Akihiko Tsujibata4 and Masaki Fujimura2 1 Department of Respiratory Medicine, Komatsu Municipal Hospital, Komatsu, 2Department of Respiratory Medicine, Cellular Transplantation Biology, Kanazawa University Graduate School of Medicine, Kanazawa, 3Department of Surgery and 4Department of Pathology, Komatsu Municipal Hospital, Komatsu, Japan. Accepted 13 January 2012 Correspondence to: Satoshi Watanabe, Department of Respiratory Medicine, Komatsu Municipal Hospital, Ho-60, Mukaimoto-ori-machi, Komatsu #923-8560, Japan. E-mail: [email protected]

References 1 Hatron PY, Tillie-Leblond I, Launay D et al. Pulmonary manifestations of Sjo¨gren’s syndrome. Presse Med 2011; 40:e71–86. 2 Kurumagawa T, Kobayashi H, Motoyoshi K. Potential involvement of subclinical Sjo¨gren’s syndrome in various lung diseases. Respirology 2005;10:86–91. 3 Vitali C, Bombardieri S, Jonsson R et al. Classification criteria for Sjo¨gren’s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 2002;61:554–8. 4 Koyama M, Johkoh T, Honda O et al. Pulmonary involvement in primary Sjo¨gren’s syndrome: spectrum of pulmonary abnormalities and computed tomography findings in 60 patients. J Thorac Imaging 2001;16:290–6.

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5 Ichikawa Y, Kinoshita M, Koga T et al. Lung cyst formation in lymphocytic interstitial pneumonia: CT features. J Comput Assist Tomogr 1994;18:745–8. 6 Kobayashi H, Matsuoka R, Kitamura S et al. Sjo¨gren’s syndrome with multiple bullae and pulmonary nodular amyloidosis. Chest 1988;94:438–40. 7 Cantin L, Bankier AA, Eisenberg RL. Multiple cystlike lung lesions in the adult. AJR Am J Roentgenol 2010;194:W1–11.

Rheumatology 2012;51:1336–1338 doi:10.1093/rheumatology/kes012 Advance Access publication 23 February 2012

Hyperferritinaemia and macrophage activation in a patient with interstitial lung disease with clinically amyopathic DM SIR, Clinically amyopathic DM (C-ADM) is characterized by typical skin lesions with amyopathy or hypomyopathy and was recently reported to be complicated by rapidly progressive interstitial lung disease (RP-ILD), especially in those patients who express the anti-C-ADM-140 antibody [1]. RP-ILD with a fatal outcome is commonly associated with hyperferritinaemia [2–4]. However, it has not been clarified as yet why hyperferritinaemia was present in patients with C-ADM-related RP-ILD. In the present study we demonstrated the presence of systemic ferritin-producing macrophages in a C-ADM-related RP-ILD autopsy case. A 58-year-old Japanese man was admitted with a heliotrope rash and Gottron’s papules of the MCP, elbow and knee joints. Laboratory investigations revealed that the level of serum creatine kinase was 70 IU/l, which is within normal limits, and that the KL-6 and ferritin levels were 735 U/ml (normal value